Primary chordomas, rare cancers arising from the notochord remnants, are extremely rare in the pediatric population. This study examined a large cohort of primary chordoma patients to determine ...factors impacting prognosis and survival.
Demographic and clinical data on 1,358 primary chordoma patients (86 pediatric patients ≤19 years of age and 1,272 adult patients ≥20 years of age) were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011).
Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite the majority of primary chordomas presenting with locoregional involvement (90.4%), pediatric patients had more distant disease (14.8 vs. 9.2%, p < 0.05). Survival among pediatric patients having surgery only was significantly longer than for adults (22.5 vs. 14.3 years, p < 0.001). Overall survival was longer (17.2 vs. 12.6 years) and overall mortality was lower in pediatric patients (38.4 vs. 49.8%), but cancer-specific mortality was higher (37.2 vs. 28.6%, p < 0.005).
Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite having a higher rate of metastasis, they have prolonged survival compared to adults. Surgical resection significantly improves survival in pediatric primary chordoma patients, and should be considered as first-line therapy in all eligible children.
Background: Ocular melanoma (OM) comprises <5% of all melanomas. Uveal melanoma (UM) is the most common subtype of OM, while conjunctival melanoma (CM) is rare and differs significantly from UM. The ...purpose of this study is to evaluate a large cohort of OM patients to differentiate demographic, pathologic, and clinical factors between these two neoplasms, which may affect treatment and outcomes. Methods: The Surveillance, Epidemiology, and End Results database (1973-2012) was used to extract demographic and clinical data on 8,165 OM patients (92.1% UM and 7.9% CM). Results: Both CM and UM were most prevalent among Caucasian males in the seventh decade of life. UM patients presented more often with localized disease (90.9% vs 81.2, P<0.01). Surgery (42.8%), radiation (43.0%), or combined surgery and radiation (7.0%) were used in the treatment of UM, while CM was treated almost exclusively with surgery (88.7%). Mean overall survival was longer (15.4 vs 14.6 years; P<0.01) and mortality rates were lower in patients (38.8% vs 46.1%; P<0.01) with CM. Conclusion: Despite presenting with more advanced disease than UM, CM is associated with an increased overall survival. Surgery is the primary therapy for CM, whereas radiotherapy is the primary therapy for UM and is associated with prolonged survival. Keywords: ocular melanoma, uveal melanoma, conjunctival melanoma, SEER
Introduction. Hepatocellular carcinoma (HCC) is a rare pediatric cancer accounting for 0.5% of all pediatric malignancies. This study examines a large cohort of HCC patients in an effort to define ...the factors impacting clinical outcomes in pediatric HCC patients compared to adults. Methods. Demographic and clinical data on 63,771 HCC patients (257 pediatric patients ≤ 19 and 63,514 adult patients age greater than or equal to 20) were abstracted from the SEER database (1973-2011). Results. HCC was more common among males (59.5% pediatric and 75.1% adults) and Caucasians (50.4% and 50.5%), p < 0.05. Children more often presented with fibrolamellar variant HCC (24.1% versus 0.3%, p = 0.71) and advanced HCC, including distant disease (33.1% versus 20.8%, p < 0.001), and tumors > 4 cm in size (79.6% versus 62.0%, p = 0.02). Pediatric HCC patients undergoing surgery (13.107 versus 8.324 years, p < 0.001) had longer survival than adult HCC patients. Overall mortality was lower (65.8% versus 82.0%, p < 0.001) in the pediatric HCC group. Conclusion. HCC is a rare pediatric malignancy that presents most often as an advanced tumor, >4 cm in Caucasian males. Children with HCC achieve significantly longer mean overall survival compared to adults with HCC, primarily attributable to the more favorable fibrolamellar histologic variant, and more aggressive surgical intervention, which significantly improves survival.
Completion pancreatectomy (CP) is a reoperative procedure to excise remnant pancreatic tissue after a prior pancreatic resection. In this study, we document our institution’s experience with CP for ...recurrent malignant disease of the pancreas, describing indications for surgery, procedures performed, and patient outcomes.
We performed a retrospective review of 861 patients from the pancreatic surgery database in the Department of Surgery of Thomas Jefferson University from October 2005 to December 2010 to identify all cases of CP performed for suspected malignant disease.
Eleven patients underwent reoperative CP at our institution from 2005 to 2010. The median time interval between the initial operation and CP was 32 mo. A combination of clinical symptoms, elevated tumor markers, and imaging studies were used for diagnosis of recurrent disease. Pancreatic ductal adenocarcinoma was the most common pathology, found in six patients. The postoperative complication rate was 18% and the median postoperative hospital length of stay was 6 d. There were no 30-d readmissions and no perioperative deaths. The 1-y survival rate following CP was 71% with an overall median survival of 17.5 mo.
CP is a safe and effective option for a highly selected group of patients with suspected recurrent malignant disease of the remnant pancreas. Morbidity and mortality rates are within acceptable limits and similar to initial pancreatic resection. Eligibility depends heavily upon the absence of distant metastatic disease, technical factors for resection, and patient performance status.
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